Takayasu arteritis is a rare autoimmune disease affecting large and moderate sized arteries, often involving the aorta or coronary vasculature. We report a case of an adolescent male with a history of recurrent respiratory tract infections who presented w
ith fever, cough, and shortness of breath and who was diagnosed with acute aortic valve failure and coronary ischemia. Ultimately, the patient's condition was attributed to Takayasa arteritis. This typical presentation of an atypical disease provides valu
able teaching points, including the use of bedside echocardiography for the diagnosis of acute aortic insufficiency and the differential diagnosis of increased erythrocyte sedimentation rate. It also serves to remind clinicians to maintain a high index of
suspicion for unusual disease processes in patients who fail to respond to empiric therapy for recurrent subacute illnesses.

The objective of the present study was to evaluate the benefit of beta-blocker therapy for patients with Takayasu arteritis complicated by moderate or severe aortic regurgitation. Clinical and echocardiographic evaluation was performed in 20 Japanese wome
n in a follow-up period of 7.0 +/- 2.0 years. The patients were divided into 2 groups: Group A (n=10) patients who did not receive beta-blockers, and Group B (n=10) patients treated with long-term (5.1 +/- 1.6 years) therapeutic doses of beta-blockers. Le
ft ventricular wall thickness increased significantly in all Takayasu patients who did not receive beta-blockers. Consequently, a remarkable increment in left ventricular mass took place (232 +/- 59 to 361 +/- 79 g; p < 0.005). In the same group, progress
ive worsening of the symptoms, with no reduction in the percent fractional shortening, was observed in 2 patients, while reduction of this last index was present in 1 asymptomatic patient. On the other hand, among the patients who were treated with beta-b
lockers, left ventricular mass still increased in 6 cases, while it clearly decreased in the other 4 cases (290 +/- 171 to 284 +/- 61 g; NS). The increment in wall thickness or left ventricular mass observed among patients with beta-blocker therapy was cl
early less than the one registered among those who had not received beta-blockers. Furthermore, no worsening of the symptoms and/or left ventricular performance was observed during the follow-up period for patients receiving beta-blockers. We conclude tha
t beta-blocker therapy can slow and even reverse the progression of left ventricular hypertrophy in patients with Takayasu arteritis complicated by moderate or severe aortic regurgitation. The mechanism still needs to be elucidated. We believe an effectiv
e reduction in the excessive afterload imposed on the left ventricle to be most likely responsible, but cardiac beta-receptor up-regulation might also be involved. Deterioration of the clinical status and/or impairment of left ventricular function were no
t associated with beta-blocker therapy in our patients. Therefore, these agents can be used safely alone or in addition to standard anti-hypertensive therapy when attempting to reduce excessive afterload, in spite of the presence of severe aortic regurgit
ation.

Annuloaortic ectasia (AAE) associated with aortic regurgitation (AR) is a rare involvement in Takayasu arteritis. There are many difficult problems in surgical treatment of this lesion because of its inflammatory nature. We developed a composite graft rep
air technique to avoid complications associated with Bentall type procedures for treatment of AAE+AR. The technique consists of a double proximal suturing and direct coronary artery reimplantation as a Carrel patch without wrapping. Eight patients (one ma
le, seven females) received this composite graft repair for treatment of AAE+AR due to Takayasu arteritis. Of them, two patients were in active phase. There was one hospital death probably due to ventricular arrhythmia, however, no late death occurred. An
d there were no late complications related to operative procedures. Thus, our composite graft repair technique for treatment of AAE+AR in Takayasu arteritis can prevent major complications associated with Bentall type procedures. However, close serial fol
low-up, careful control of inflammation, and early detection and treatment of other vascular involvements are necessary in the postoperative management of patients with Takayasu arteritis.

A 37-year-old non-smoking woman was admitted to our hospital because of nocturnal coughing and exertional dyspnea after using an ultrasonic humidifier. Chest roentgenograms and computed tomography showed many ground-glass opacities in the right upper lobe
and left upper lung field. Her symptoms were alleviated and erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels returned to normal following the administration of prednisolone (40 mg/day). The same symptoms subsequently recurred, and
ESR and CRP levels increased proportionately to a decreased dosage of prednisolone. In addition, chest computed tomography showed new opacities in the left lower lobe. Transbronchial lung biopsy specimens revealed multiple stenotic or obstructive changes
in the small pulmonary arteries by organizing thromboemboli, with no vasculitis. The diagnosis of chronic thromboembolic pulmonary hypertension was made on the basis of multiple defects from lung perfusion scintigraphy, irregular or tapering of the pulmo
nary arteries detected by pulmonary arteriograms, and increased pulmonary pressure measured by echocardiography. We propose that the pulmonary thromboembolism observed in this case was related to the initial pulmonary involvement of Takayasu arteritis, si
nce the patient's symptoms, ESR and CRP levels all improved markedly following the administration of prednisolone.

We prospectively performed the follow-up study in 11 female patients with Takayasu arteritis and severe aortic regurgitation by echocardiography. A mean follow-up period was 4 years. The inflammatory state was controlled in all patients. Antihypertensive
agents including beta-blocker were administered in nine patients. Heart failure did not progress in all patients except one. No candidate for cardiac surgery appeared during the follow-up period. Aortic root diameter, left atrial, left ventricular end-dia
stolic and end-systolic dimensions, wall thickness, left ventricular mass, and percent fractional shortening of the left ventricle showed no significant change in echocardiography. These data indicate that left ventricular disturbance might be slowly prog
ressive in patients with Takayasu arteritis and severe aortic regurgitation. Systemic hypertension and the inflammatory state should be well controlled in managing the patients. beta-blocker might be useful in some patients with Takayasu arteritis and sev
ere aortic regurgitation. Further follow-up is necessary for the decision of the cardiac surgical indication.

A 36-year-old woman was delivered to our hospital with suspected aortic dissection from an outlying hospital in May 1994. She reported a history of acute and persistent thoracic and epigastric pain. The physical examination revealed a minor senso-motorica
l palsy of the left side as residuum after minor strokes occurring 12/93 and 4/94. Also, a marked hypertension (170/100 mm Hg) was present. The hematologic and blood chemical values were normal with a white cell count of 12,000, an erythrocyte sedimentati
on rate (ESR) of 19 mm/h and a c-reactive protein (CRP) of 2.2 mg/dl. The electrocardiogram was normal. Transthoracic- and transesophageal-echocardiography (TTE, TEE) revealed an eccentric thickening of the whole wall of the descending aorta up to the bif
urcation with a stenosis at the side of the diaphragma. Those findings were confirmed by computed tomography. Because of the acute onset of symptoms and the results of the imaging procedures aortic dissection de Bakey type III was diagnosed and the patien
t was treated with beta-blockers. Symptoms were relieved over the following days. After 2 days a pleuric effusion developed and all inflammatory tests rose (fibrinogen 780 mg/dl, ESR 80 mm/hr, CRP 16 mg/dl). At this time the differential diagnosis of an a
rteritis was considered. A new TEE-study demonstrated no change, but now more attention was given to the fact that no dissection membrane could be visualized and all wall structures were thickened. In combination with the history of cerebral infarction du
e to carotid obstruction and the elevated laboratory values the diagnosis of Takayasu-aortitis was established and corticoid therapy was started. Within a few weeks the arterial changes diminshed markedly and the woman became free of symptoms. This case d
emonstrates the rare situation of an arteritis mimicking aortic dissection, in which the clinical course revealed the true diagnosis.

Although aortic regurgitation (AR) in patients with Takayasu arteritis (TA) is believed to be caused by dilatation of the aortic ring or thickening of the aortic cusps, the pattern of left ventricular hypertrophy (LVH) in these patients has not been descr
ibed. To clarify the geometry of the LV in patients with TA that had been documented both echocardiographically and hemodynamically, the authors evaluated 22 patients with TA and severe AR (group 1), 10 with TA and systemic hypertension (group 2), and 17
with isolated severe AR of other etiologies (group 3). M-mode echocardiography showed that LV dimensions were smaller in groups 1 and 2 than in group 3 and that wall thickness was greater in group 1 than in group 3. The concentric hypertrophic ratio (CHR
= wall thickness/LV end-diastolic dimension+wall thickness) was larger than group 1 (p < 0.001) and group 2 (p < 0.01) than in group 3. CHR was similar in groups 1 and 2. Systolic blood pressure was significantly increased in group 2 and showed a tendency
to increase in group 1 as compared with group 3. The results confirmed the presence of concentric LVH related to a pressure overload in patients with TA, even when TA was complicated by severe AR.

To characterize aortic regurgitation in patients with Takayasu arteritis, we studied 48 females with arteritis (mean age 47 +/- 12 years) by means of color Doppler echocardiography. Aortic regurgitation was confirmed in 32 out of 48 patients (67%) by colo
r-flow mapping. Twenty-four patients had mild or no aortic regurgitation (group A), 9 had moderate (group B), and 15 had severe (group C) aortic regurgitation. We compared the echocardiographic data obtained from patients with Takayasu arteritis with thos
e of 14 normal controls and 9 patients with severe aortic regurgitation of valvular origins (group V). The aortic root diameter (AOD) in group B (23 +/- 4 mm/M2) and group C (22 +/- 3 mm/M2) revealed a statistically significant large value as compared wit
h that in group A (18 +/- 2 mm/M2) and normal controls (17 +/- 3 mm/M2). However, the differences, between groups B and C and groups C and V, were not significant. The AOD was not obviously dilated in a considerable number of group C patients. Aortic valv
e involvement was seen in several group C patients and moderate concentric left ventricular hypertrophy was present in all group C patients. Group C patients therefore, have concentric left ventricular hypertrophy but may or may not have dilatation of the
aortic root which can be detected on echocardiography. We conclude that aortic valve involvement may cause aortic regurgitation in some patients with Takayasu arteritis and that aortic regurgitation is more common than previously believed.

Cardiology. 1992;80(3-4):180-3.
Left ventricular geometry in Takayasu arteritis complicated by severe aortic regurgitation.
Hashimoto Y, Numano F, Oniki T, Shimizu S.
Third Department of Internal Medicine, Tokyo Medical and Dental University, School of Medicine, Japan.
Although it has been reported that the aortic regurgitation (AR) of patients with Takayasu arteritis is due to dilatation of the aortic ring, the geometry of the left ventricle (LV) has not been described. We compared the cardiac findings in patients with
Takayasu arteritis (TA) and severe AR with those of patients having severe AR of other origins. Echocardiographically, wall thickness (WT) and the concentric hypertrophic ratio (WT/WT + left ventricular end-diastolic dimension) were greater in patients w
ith TA. It is concluded that the LV of the TA patients revealed concentric hypertrophy even when AR was severe.

The most severe arteritis due to Takayasu's disease are those related to renal and coronary arteries. The first one because it produces severe arterial hypertension and the second one because it puts the patient in high risk of suffering either myocardial
ischemia or infarction. These situations worsen when this entity is associated to valvular heart lesions. The authors present the clinical cases of two female patients with Takayasu's disease. One of them in acute phase of the illness, where coronary art
eritis, mild coarctation of the aorta, right pulmonary artery stenosis, and pulmonary valve stenosis were present. The second patient was seen during the remission phase of the disease with obstruction of the left subclavicular artery, renal arteritis, se
vere arterial hypertension and aortic valve insufficiency. The authors discuss the prognosis of patients with Takayasu's disease associated to valvular heart disease and its role in the etiology of pulmonary valvular stenosis. Finally, the authors point o
ut the importance of recognizing the active and non active phases of the Takayasu's disease in relation of the adequate stage for surgical treatment of the lesions caused by this disease.